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Transmastoid decompression of the facial nerve in temporal bone fracture.

Among 41 cases of facial palsy caused by closed heart injury, temporal bone fractures were surgically confirmed in 36 cases, of which there were two mixed fractures and 34 longitudinal fractures. The fracture involved the geniculate ganglion area in 20 cases (55%). In 15 cases, decompression of the facial nerve was carried out using the transmastoid supralabyrinthine approach with disarticulation of the incus; in only five cases was the middle fossa approach used. The technique avoids the craniotomy for the middle fossa approach and is a reliable method of treatment in the majority of patients with facial palsy caused by temporal bone fracture involving the geniculate ganglion area.

Facial Nerve↗

Congenital conductive hearing loss in Apert syndrome.

Acrocephalosyndactyly (Apert syndrome) is a rare craniosynostotic syndrome characterized by acrocephaly, syndactyly of the hands and feet, and--occasionally--conductive hearing loss. We report three cases of conductive hearing loss in Apert syndrome. One patient was found to have bilateral stapes fixation. His daughter (the second case) had chronic bilateral otitis media with effusion. The third case involved a fixed incus and hypomobile stapes. The management of these patients and a review of the literature are presented.

Acrocephalosyndactylia↗

Hearing results with clothespin ossiculoplasty: preliminary report on the Kraus Modified Schuring Ossicle-Cup Prosthesis (Clothespin Prosthesis).

The clothespin partial ossicular replacement prosthesis (PORP) is designed to increase joint stability during incus replacement ossiculoplasty. Fundamental modifications have been made in the Schuring ossicle-cup, which include a forked well, increased well wall thickness and length, and a flexible tip disk added to the shaft. The forked well functions like a straight clothespin rather than an inverted cup. The forked well enables the clothespin prothesis to slide down over the stapes superstructure, between the facial nerve canal and the promontory, to form a stable, mortise-and-tenon prosthesis-stapedial joint. Joint stability is enhanced because the inferior tine of the forked well is able to lever against the inferior surface of the stapes superstructure, creating a counterforce to gravity. This is in contrast to the more unstable ball-and-socket joint created by most partial ossicular replacement prostheses when they articulate with the stapes capitulum. The addition of a flexible disk to the shaft tip produces a tight, stable union between the prosthesis and the ossicular cap by increasing resistance at the shaft-ossicle interface. Fluoroplastic composition maximizes intraoperative versatility and reliability while the ossicle cap minimizes extrusions. One-year hearing results for twelve chronic ear patients with mobile stapes undergoing clothespin ossiculoplasty during intact canal wall tympanomastoidectomy revealed postoperative air-bone gaps within 20 dB in 92% of cases. The mean postoperative air-bone gap was 8.9 dB, and the mean improvement in air-bone gap was 14.4 dB.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Results of revision stapedectomy for conductive hearing loss.

Revision stapedectomy operations performed over a 13-year period (1977 to 1990) for a conductive hearing loss are reviewed in terms of intraoperative findings and hearing results. All operations were performed in a conventional manner without use of laser techniques. A management algorithm based on intraoperative findings is described. Results are compared with previously reported series. The 66 cases include 20 males and 46 females, ranging in age from 8 to 73 years. Mean time between original and revision stapedectomy was 12.5 years. Prostheses encountered at time of revision included wireloop (29), Robinson (18), polyethylene (14), other (3), and two were not found. The most common cause of failure was displacement of the prosthesis. Incus erosion was found in 48% of wireloops, 35% of polyethylene, and only 11% of the Robinson prostheses. Revision resulted in closure of the pure-tone average (PTA) air-bone gap to within 10 dB in 46% and to within 15 dB in 76%. Sensorineural hearing loss (> 10 dB) occurred in 5 cases (7.6%), and a decline in speech discrimination (> 10%) occurred in 17%. Three of four cases requiring drillout had gap closure to within 10 dB. Findings suggest that our management technique produces results comparable to other large reported series. Drillout at the time of revision can be recommended.

Adolescent↗

Effect of changes in mass on middle ear function.

Vibrating systems such as the middle ear are affected by changes in mass. After disease or ear surgery, significant changes in mass may contribute positively or negatively to the postoperative hearing threshold. This article describes experiments in 15 human temporal bones of the addition or reduction of mass on the middle ear transfer function. Measurement of stapes and umbo vibration was performed using a Laser Doppler Vibrometer before and after the addition of different masses at several sites on the tympanic membrane (TM) and ossicular chain. The input was 61 pure tones swept from 147 to 19433 Hz at 80 dB SPL. The addition of mass onto the TM produced varying detrimental effects on sound transmission, depending on the location and amount of mass. The insertion of ventilation tubes, weighing 12 to 17 mg each, produced losses at 1.5 to 5.0 kHz compared with tympanotomy alone. Addition of mass to the umbo and malleus head produced a loss at mid and high frequencies, whereas addition of mass on the incus long process and stapes also produced a high-frequency decrease in stapes displacement. Reduction of TM mass by removal of the epithelium produced an increase, especially at 2.0 to 4.0 kHz.

Adult↗

Acute unilateral hearing loss as an unusual presentation of cholesteatoma.

BACKGROUND: Cholesteatomas are epithelial cysts that contain desquamated keratin. Patients commonly present with progressive hearing loss and a chronically discharging ear. We report an unusual presentation of the disease with an acute hearing loss suffered immediately after prolonged use of a pneumatic drill. CASE PRESENTATION: A 41 year old man with no previous history of ear problems presented with a sudden loss of hearing in his right ear immediately following the prolonged use of a pneumatic drill on concrete. The cause was found to be a fractured long process of incus which had been eroded by the presence of an attic cholesteatoma. A tympanomastoidectomy and ossiculoplasty was performed with good result. CONCLUSION: Cholesteatomas may be asymptomatic and insidious in their onset. This case illustrates the point that an indolent disease such as this may present in unusual ways and the clinician must always have a high index of suspicion combined with thorough assessment and examination of every patient.

Journal Article↗

Murine teratology of fluconazole: evaluation of developmental phase specificity and dose dependence.

The potential of in utero exposure to fluconazole to initiate teratogenesis was analyzed in ICR (CD-1) mice. Developmental phase specificity was determined by treating mice with single oral doses of 700 mg/kg on gestational day 8, 9, 10, 11, or 12. Control animals received vehicle on gestational days 8-12. Gestational day 10 was identified as the phase of maximal sensitivity for induction of cleft palate, the predominant teratogenic effect induced by fluconazole, with 50% of fetuses exposed on this developmental phase being affected. After treatments on gestational day 8, 9, 11, or 12, cleft palate occurred with lower frequencies: 12, 21, 28.7, and 2.7%, respectively. Examination of skeletal morphology revealed anomalies of the middle ear apparatus in 15% of the fetuses that were exposed on gestational day 8. Dysmorphic tympanic ring and absence of the incus were the more common ear anomalies recorded. Reduced humeral length was noted in 22% of fetuses that were exposed on gestational day 10. Dose-response relationship was investigated by treating animals with 0 (vehicle), 87.5, 175, or 350 mg/kg on gestational day 10, coincident with the phase of peak teratogenic sensitivity. Besides showing that fluconazole operates under a strict dose-response mechanism, the study identified 175 mg/kg as the lowest observed adverse effect level for cleft palate induction, with 7.6% of the exposed fetuses being affected.

Abnormalities, Drug-Induced↗

Assembling a functional tympanic membrane: signals from the external acoustic meatus coordinate development of the malleal manubrium.

In terrestrial mammals, hearing starts with the perception of acoustic pressure by the tympanic membrane. Vibrations in this membrane are then transduced into the inner ear by the ossicle chain of the middle ear, composed of the malleus, incus and stapes. The proper connection of the ossicle chain with the tympanic membrane, provided by the insertion of the manubrium of the malleus into the eardrum, is essential for the functionality of the hearing apparatus. We describe here the mechanisms regulating the development of the manubrium and its integration into the tympanic membrane. We show that the external acoustic meatus (EAM), which eventually forms the outer epithelium of the tympanic membrane, plays an essential role in this developmental process. Histological and expression analyses indicate that the manubrium develops close to the EAM with a similar temporal sequence. In addition, when the middle ear ossicles are allowed to develop in vitro under conditions that do not support further EAM development, the manubrium develops only up to the stage of its induction at the time of explantation. Moreover, genetically or teratogenically derived alterations in the EAM also have an effect on manubrial development. Finally, we show that the EAM is the source of two quite opposite activities, one that induces chondrogenesis and another that represses it. The combination of these two activities results in the proper positioning of the manubrium.

Animals↗

Does it make sense to preserve the stapedial muscle during surgical treatment for otosclerosis?

Our study aimed to show whether it is useful to preserve the stapedial muscle during the surgical treatment of otosclerosis. The study was carried out on 67 patients with tympanoscopic evidence of otosclerosis. In group A (34 patients) the stapedial muscle was intra-operatively preserved as follows: the stapedial suprastructure with preserved muscle tendon was transpositioned onto the longer arm of the incus and secured with a wire loop. The mean uncomfortable level for sounds was measured after surgery and the results of group A patients were compared with those obtained in group B (33 patients, who underwent standard surgery). One month after surgery, in group A patients, the cochleostapedial reflex was evoked in 21 patients (61.8 per cent) and two months later in 32 patients (94.1 per cent). One month after operation, in group A patients the mean uncomfortable level was 108.8 dB, while three months later it increased to 114.1 dB. In group B, the relevant values were 97.8 dB one month after surgery, and 98.0 dB three months later. Our presumption that stapedial muscle preservation is necessary has been proved by the fact that the patients with the preserved muscle had a higher noise discomfort threshold.

Adult↗

Long-term middle-ear effects of middle-ear/eustachian tube packing after translabyrinthine surgery.

The purpose of this study was to evaluate the long-term effect on middle ear and eustachian tube function following the packing of the middle-ear space to prevent cerebrospinal fluid rhinorrhoea at the termination of translabyrinthine vestibular schwannoma removal. This was an observational study of a sample of 14 patients examined between two and five years post-operatively. Photo-otoscopy and tympanometry were performed bilaterally to evaluate appearance and function. All operated middle ears were air-containing although eight out of 14 showed scarring as evidence of operative intervention. There were no drum retractions. Ten out of 14 middle-ear compliance peaks were lower in the operated ear with four flat traces. Two out of 14 were the same and two out of 14 showed high compliance. The middle-ear pressure was always within the normal range when determinable. This technique appears to have no detrimental effects on the middle ear and does not permanently obliterate the eustachian tube. The reduced compliance in the majority of cases is probably a result of scarring and fibrosis. When drum hypermobility is seen this is accounted for by incus removal.

Acoustic Impedance Tests↗

Middle-ear reconstruction: a review of 150 cases.

This study is based on a retrospective analysis of 150 cases of tympanoplasty and ossicular chain reconstruction as a one-stage procedure. It was conducted at the Federal Government Services Hospital, Islamabad from 1983 to 1999. Temporalis fascia was used for myringoplasty and a sculptured autologous incus to bridge the malleus to stapes head and malleus to footplate gap. These interpositions have produced stable ossicular assemblies and provided satisfactory post-operative hearing gains. One hundred and twenty-six (84 per cent) patients achieved a healed tympanic membrane three months post-operatively. One year post-operatively, 121 (81 per cent) patients had an intact tympanic membrane and at five years 119 (79 per cent) had an intact tympanic membrane. The success rate was based on an intact tympanic membrane and an air-bone gap of less than 30 dB one-year post-operation. Accordingly, the overall success rate was 80 per cent (120 cases). Hearing gains in cases with an absent stapes arch were poorer than in cases with an intact arch. Early (one to three months) post-operative complications included tympanic membrane perforation, lateralization of the graft and persistence of infection. Late (one to five years) complications included the appearance of an anterior perforation and retraction and thinning of the tympanic membrane.

Adolescent↗

Revision surgery for chronic otitis media: recurrent-residual disease and hearing.

The aim of this study was to determine the effect of surgical approach, intact canal wall (ICW) or canal wall down (CWD), upon the success of revision surgery for chronic otitis media (COM). A retrospective analysis of 367 patients (including 65 children aged <15 years) who underwent revision tympanoplasty because of persistent disease was performed. Single-staged tympanoplasty was performed, preserving the canal wall when present. Hearing was reconstructed with allograft incus. Follow-up ranged from one to 15 years. Hearing was determined by pre- and post-operative air-bone gaps. Post-operative re-perforation, aural discharge and/or cholesteatoma rates were similar for CWD and ICW. Cholesteatoma could present following the revision, even though it was not apparent at surgery. Following tympanoplasty, the final hearing was not significantly affected by the surgical approach or presence of cholesteatoma. Improvement in hearing was adversely affected by cholesteatoma or an absent stapes suprastructure. Revision ICW and CWD operations were both successful in controlling signs of COM. Cholesteatoma is a peripheral risk in COM and may become apparent after revision surgery.

Adult↗

Myringostapediopexy: surgical expectation.

Myringostapediopexy may occur as a result of incus erosion with medialization of the tympanic membrane and is recognized as often producing serviceable hearing. The technique may be used as part of tympanoplasty following either canal wall up or canal wall down surgery for chronic otitis media. The use of this type of reconstruction is influenced by the anatomy of the ear after disease excision. This review of the hearing levels associated with myringostapediopexy shows that there is a similar range of hearing level both for naturally formed as well as surgically fashioned myringostapediopexy. For both 'naturally formed' and following canal wall up surgery about 80 per cent of patients will have an air-bone gap of 20 dB or less compared to 60 per cent of those who undergo canal wall down surgery.

Adolescent↗

Anomalies of the auditory organ in trisomy 18 syndrome: human temporal bone histopathological study.

The purpose of this study was to define the histopathological changes in the temporal bone of a fetus with trisomy 18 syndrome, a stillborn due to perosplanchnia. Several anomalies were found including malformation of the auditory ossicles, residual mesenchyme in the middle ear, aberrant tensor tympani muscle, absence of stapedial tendon, aberrant lateral ampullary nerve and wide endolymphatic sinus. The incus body was deformed and separated from the long process by connective tissue and monocrural stapes was noted in the right ear. Three-dimensional reconstruction images provided a clear view of the auditory ossicle malformation. The abnormal findings in our case indicate that ear anomalies in this syndrome might be derived from the component around the first and second branchial arches.

Chromosomes, Human, Pair 18↗

Combined Heermann and Tos (CHAT) technique in cholesteatoma surgery: surgical technique and preliminary results.

The combined Heermann and Tos (CHAT) technique is the combination of Heermann's 'cartilage palisade tympanoplasty' and Tos's 'modified combined approach tympanoplasty = modified intact canal wall mastoidectomy'. The first author (Cem Uzun) performed the CHAT technique as a one-stage operation in 15 ears of 15 patients with cholesteatoma. Two patients (one with a follow up of less than six months and one who did not show up at the final re-evaluation) were excluded from the study. Median age in the remaining 13 patients was 37 years (range: 14-57 years). Cholesteatoma type was attic, sinus (Tos tensa type 1) and tensa retraction (Tos tensa type 2) in six, five and two ears, respectively. Cholesteatoma stage was Saleh and Mills stage 1, 2, 3, 4 and 5 in one, three, four, four and one ear, respectively. The eustachian tube was not involved with cholesteatoma in any ear. After drilling of the superoposterior bony annulus, transcanal atticotomy with preservation of thin bridge and cortical mastoidectomy with intact canal wall, the cholesteatoma was removed, and the eardrum and atticotomy were reconstructed with palisades of auricular cartilage. Type I tympanoplasty was performed in two ears, type II in nine ears and type III (stapes absent) in two ears, with either autologous incus (eight cases), cortical bone (two) or auricular cartilage (one). No complication occurred before, during or after surgery. Oto-microscopy and audiometry were done before and at a median of 13 months after surgery (mean 14 months, range 7-30 months). There was no sign of residual or recurrent cholesteatoma in any patient during the follow-up period. At the final examination, all ears were dry and had an intact eardrum except one with a small, central hole, which had been seen since the early post-operative period. Clean and stable attic retraction with a wide access was observed in two ears. Post-operative hearing at the final evaluation was better (change > 10 dB) than the pre-operative one in nine ears and did not change in the remaining four. Pre- and post-operative mean hearing values were, pure-tone average 47 and 35 dB (p = 0.01) and air-bone gap 30 and 20 dB (p = 0.02), respectively. With the CHAT technique, cholesteatoma can be completely and safely removed from the middle ear, and a durable and resistant reconstruction of the middle ear with reasonable hearing can be achieved. However, a further study should analyse long-term results of a larger patient group.

Adolescent↗

Mesotympanic sarcoidosis.

Sarcoidosis often presents with signs and symptoms within the head and neck. In this region, it most commonly affects the nose, larynx, parotid and cervical lymph nodes. Within the ear, it usually causes sensorineural hearing loss of varying severity. We report a patient in whom sarcoidosis involved the middle ear and presented with a conductive hearing loss. Granulomata were found in the middle ear during an exploratory tympanotomy. The granulomatous process had caused necrosis of the long process of incus and was also encasing the chorda tympani nerve. Histological examination confirmed the diagnosis.

Ear Diseases↗

Traumatic perilymph fistula in infants: a case report.

Traumatic perilymph fistula is reported to be rare in infants because of the small size of the infant external meatus. We treated an infant with a traumatic perilymph fistula in the right ear. A metallic wire had penetrated the tympanic membrane. Horizontal-rotatory nystagmus was also observed. Computed tomographic images revealed dislocation of the ossicles. The perilymph fistula was closed under general anesthesia. The incus-stapes joint was separated and the footplate of the stapes was dislocated. Leakage of the perilymph fluid was apparent from the oval window and this fistula was closed with connective tissue. The perforation of the tympanic membrane was closed with temporal fascia. After surgery, the spontaneous nystagmus disappeared. The patient is under observation as an outpatient and is growing normally.

Child, Preschool↗

Ossicular fixation to the tegmen.

Two surgical techniques are presented for the treatment of ossicular fixation of the incus and malleus to the tegmen. One involves an atticotomy approach, the second a mastoid approach to the attic.

Ear Ossicles↗